Piel y tubo digestivo: dermatitis herpetiforme y enfermedad celíaca / Small bowel and skin: celiac disease and dermatitis herpetiformis

La dermatitis herpetiforme, también denominada Enfermedad de Dühring-Brocq, es una dermatosis autoinmune crónica que evoluciona por brotes, caracterizada por la presencia de ampollas pequeñas que tienden a agruparse, en codos, rodillas y glúteos, con disposición simétrica, intensamente pruriginosas. Es considerada una manifestación cutánea de la enfermedad celíaca. Afecta a adultos jóvenes (20 a 50 años). El estudio histopatológico evidencia ampollas subepidérmicas. La inmunofluorescencia directa es característica: depósitos granulares de IgA en las puntas de las papilas dérmicas. Aún ante falta de sintomatología digestiva debe investigarse enfermedad celíaca en todos los pacientes. La dieta libre de gluten es la clave del tratamiento. En aquellos pacientes con intenso prurito o con una dermatosis muy extensa se puede utilizar dapsona vía oral, que alivia rápidamente las manifestaciones cutáneas, pero no modifica el curso de la enfermedad digestiva. Se presenta un paciente en quien a partir de las lesiones cutáneas se realizó diagnóstico de dermatitis herpetiforme primero y de enfermedad celíaca luego

Dermatitis herpetiformis, also known as Dühring-Brocq disease, is a chronic autoimmune dermatosis that evolves in outbreaks. It is characterized by the presence of small blisters that tend to cluster on the elbows, knees, and buttocks, with a symmetrical distribution and intense itching. It is considered a cutaneous manifestation of celiac disease. It affects young adults (20 to 50 years old). Histopathological examination reveals subepidermal blisters. Direct immunofluorescence is characteristic, showing granular deposits of IgA at the tips of the dermal papillae. Even in the absence of digestive symptoms, celiac disease should be investigated in all patients. A gluten-free diet is the key to treatment. In patients with intense itching or extensive dermatosis, oral dapsone can be used to quickly relieve cutaneous manifestations, but it does not alter the course of the digestive disease. We present a patient in whom the diagnosis of dermatitis herpetiformis was made initially, followed by a diagnosis of celiac disease based on the skin lesions

The importance of direct immunofluorescence in pemphigus herpetiformis diagnosis

Abstract Pemphigus herpetiformis is an autoimmune bullous disease, that combines clinical features of dermatitis herpetiformis and linear IgA bullous dermatosis and immunological characteristics of pemphigus, which makes this disease peculiar and this diagnosis rarely suspected in the first evaluation of the patient. The reported case is of a patient with clinically bullous disease similar to dermatitis herpetiformis, whose multiple biopsies were inconclusive, and only after direct immunofluorescence with a pemphigus pattern (intraepidermal intercellular pattern) the confirmation of the diagnosis was possible.

Dermatitis herpetiformis: pathophysiology, clinical presentation, diagnosis and treatment

Researches on DH have shown that it is not just a bullous skin disease, but a cutaneous-intestinal disorder caused by hypersensitivity to gluten. Exposure to gluten is the starting point of an inflammatory cascade capable of forming autoantibodies that are brought to the skin, where they are deposited, culminating in the formation of skin lesions. These lesions are vesico-bullous, pruritic, and localized especially on elbows, knees and buttocks, although atypical presentations can occur. Immunofluorescence of perilesional area is considered the gold standard for diagnosis, but serological tests help in cases where it is negative. Patients who follow glutenfree diets have better control of symptoms on the skin and intestine, as well as lower risks of progression to lymphoma. Dapsone remains the main drug for treatment, but it requires monitoring of possible side effects, some potentially lethal.

Pustular psoriasis of pregnancy (Impetigo herpetiformis) - case report / Psoriase pustulosa da gestacao (Impetigo herpetiforme) - relato de caso

Impetigo herpetiformis is a rare dermatosis of pregnancy with typical onset during the last trimester of pregnancy and rapid resolution in the postpartum period. Clinically and histologically, it is consistent with pustular psoriasis. This similarity has led some authors to name the disease "the pustular psoriasis of pregnancy". We report the case of a patient who developed impetigo herpetiformis in two sucessive pregnancies.

Impetigo herpetiforme é uma dermatose gestacional rara que se inicia tipicamente durante o último trimestre e evolui com rápida resolução no período pós-parto. Clinica e histologicamente é consistente com psoríase pustulosa. Essa similaridade tem levado alguns autores a nomearem a doença como "psoríase pustulosa da gestação". Relatamos o caso de uma paciente que apresentou impetigo herpetiforme em duas gestações subsequentes.

Dermoscopy examination of petechial lesions in a patient with Dermatitis Herpetiformis / Exame dermatoscopico de lesoes petequiais num paciente com Dermatite Herpetiforme

An uncommon skin manifestation of Dermatitis Herpetiformis is palmar and plantar purpura. Dermoscopic examination is useful for any skin condition since it allows recognition of structures that are not discernible to the naked eye. A 22 year-old Caucasian man was admitted with excoriated lesions and pruritus. Petechial lesions could be seen on volar aspect of the digits on the hands and feet. Dermoscopy examination revealed erythematous and violaceous dots and erythematous and brown dots.

Púrpura palmar e plantar é uma manifestação cutânea incomum da Dermatite Herpetiforme. A dermatoscopia é útil para o exame dermatológico por permitir o reconhecimento de estruturas que ao olho nu não são perceptíveis. Doente caucasiano masculino de 22 anos que queixava-se de lesões escoriadas e prurido. Observouse na face volar dos dedos dos pés e das mãos lesões petequiais. O exame dermatoscópico revelou pontos eritematosos e violáceos, além de pontos eritematosos e marrons.

Review: dermatitis herpetiformis / Revisao: dermatite herpetiforme

Dermatitis herpetiformis (DH) or Duhring-Brocq disease is a chronic bullous disease characterized by intense itching and burning sensation in the erythematous papules and urticarial plaques, grouped vesicles with centrifuge growth, and tense blisters. There is an association with the genotypes HLA DR3, HLA DQw2, found in 80-90% of cases. It is an IgA-mediated cutaneous disease, with immunoglobulin A deposits appearing in a granular pattern at the top of the dermal papilla in the sublamina densa area of the basement membrane, which is present both in affected skin and healthy skin. The same protein IgA1 with J chain is found in the small intestinal mucosa in patients with adult celiac disease, suggesting a strong association with DH. Specific antibodies such as antiendomysium, antireticulina, antigliadin and, recently identified, the epidermal and tissue transglutaminase subtypes, as well as increased zonulin production, are common to both conditions, along with gluten-sensitive enteropathy and DH. Autoimmune diseases present higher levels of prevalence, such as thyroid (5-11%), pernicious anemia (1-3%), type 1 diabetes (1-2%) and collagen tissue disease. The chosen treatment is dapsone and a gluten-free diet.

Dermatite herpetiforme é uma doença bolhosa crônica caracterizada por intenso prurido e sensação de queimação em pápulas eritematosas e placas urticariformes, vesículas agrupadas com crescimento centrífugo e bolhas tensas. Apresenta associação com genótipos de HLA DR3, HLA DQW2 encontrados em 80 a 90% dos casos. É uma doença cutânea mediada por IgA com depósito de imunoglobulina A em padrão granular no topo da papila dérmica na área da sublâmina densa na zona da membrana basal, presente tanto na pele lesada com em área de pele sã. A mesma cadeia J da proteína IgA1 é encontrada na mucosa do intestino delgado em pacientes com doença celíaca do adulto, sugerindo forte associação com a dermatite herpetiforme. Anticorpos específicos com anti-endomísio, anti-reculina, anti-gliadina, e recentemente identificado, o subtipo transglutaminase epidérmica e tecidual, assim como a produção aumentada da zonulina, são descritas em ambas as afecções enteropatia sensível ao glúten e a deramtite herpetiforme. Exibe depósitos de IgA em padrão granular na papila dérmica. Doenças auto-imunes exibem maior prevalência como tireoidopatia em 5 a 11%, anemia perniciosa em 1 a 3%, diabetes tipo 1 em 1 a 2% e doença do colágeno. O tratamento de escolha é a dapsona e dieta isenta de glútem.

Case for diagnosis / Caso para diagnóstico

We report a clinical case of a rare variant of pemphigus - pemphigus herpetiformis - which combines the clinical features of dermatitis herpetiformis with the immunological findings of pemphigus. Due to its atypical presentation, it is frequently misdiagnosed as dermatitis herpetiformis. It is basically characterized by the herpetiform pattern of skin lesions, severe pruritus and by the presence of eosinophilic spongiosis confirmed on histopathology. We call attention to the excellent response to dapsone.

Descrevemos um caso clínico de uma variante rara de pênfigo - pênfigo herpetiforme - que combina os aspectos clínicos da dermatite herpetiforme com os achados imunológicos do pênfigo. Devido à sua apresentação atípica, é frequentemente diagnosticado equivocamente como dermatite herpetiforme. Caracteriza-se essencialmente pelo padrão herpetiforme das lesões cutâneas, prurido intenso e presença de espongiose eosinofílica no exame histopatológico. Enfatizamos a excelente resposta terapêutica à dapsona.

Lúpus eritematoso sistêmico bolhoso: diagnóstico diferencial com dermatite herpetiforme / Bullous systemic lupus erythematosus: differential diagnosis with dermatitis herpetiformis

O lúpus eritematoso sistêmico bolhoso é um subtipo raro do lúpus eritematoso sistêmico, que ocorre ainda de forma mais incomum nos pacientes pediátricos. Relatamos o caso de uma adolescente de 12 anos, apresentando lesões vésico-bolhosas em face, pescoço, tronco, mucosas oral e genital, anemia, leucocitúria estéril, FAN: 1/1280 padrão nuclear pontilhado grosso, Anti-Sm e Anti-RNP positivos. O estudo anatomopatológico sugere dermatite herpetiforme e a imunofluorescência direta revela IgG, IgA e fibrina ao longo da zona de membrana basal. Apresentamos um caso típico de lúpus eritematoso sistêmico bolhoso e enfatizamos a importância do diagnóstico diferencial com a dermatite herpetiforme.

Bullous systemic lupus erythematosus is a rare subset of systemic lupus erythematosus that is even rarer in pediatric patients. We report a case of a 12-year-old girl who presented with a vesiculobullous eruption on her face, neck, trunk and genital and oral mucosa, as well as anemia, sterile pyuria, ANA (1:1280, speckled pattern) and positive anti-Sm and anti-RNP. Pathological examination suggested dermatitis herpetiformis, and direct immunofluorescence revealed IgG, IgA and fibrin in the epithelial basement membrane zone. We present a typical case of bullous systemic lupus erythematosus and emphasize the importance of clinical and histopathological differential diagnosis with dermatitis herpetiformis.

Dermatoses bolhosas auto-imunes / Autoimmune bullous dermatoses

Dermatoses bolhosas autoimunes são doenças cuja manifestação cutânea primária e fundamental consiste em vesículas e bolhas. Classificam-se conforme a localização da bolha, em intraepidérmica e subepidérmica. Os pacientes produzem autoanticorpos contra estruturas específicas da pele detectáveis por técnicas de imunofluorescência, immunobloting e Elisa. Os recentes avanços da biologia molecular e celular têm permitido conhecer esses autoantígenos, contra os quais os pacientes se sensibilizam e que estão localizados na epiderme ou na junção dermoepidérmica. São doenças de baixa incidência, porém de elevada morbidade e por vezes letais. O objetivo deste trabalho é revisar e descrever os progressos nos conhecimentos de quatro doenças vésico-bolhosas autoimunes: pênfigo foliáceo endêmico (fogo selvagem), pênfigo vulgar, penfigóide bolhoso e dermatite herpetiforme.

Autoimmune bullous dermatoses are diseases in which blisters and vesicles are the primary and fundamental types of skin lesion. Their classification is based on the location of the blister: intraepidermal and subepidermal. Patients produce autoantibodies against self-specific structures of the skin detectable by immunofluorescence techniques, immunoblotting and ELISA. Recent advances in molecular and cellular biology have brought to knowledge these self-antigens, against which patients are sensitized, and which are found in epidermis or in the dermo-epidermal junction. These are low incidence, but high morbidity diseases that may be fatal. The aim of this article is to review and describe the progress of four autoimmune vesiculobullous disorders: endemic pemphigus foliaceous (wild fire), pemphigus vulgaris, bullous pemphigoid and dermatitis herpetiformis.

Celiac disease serology in dermatitis herpetiformis: which is the best option for detecting gluten sensitivity? / Serología de enfermedad celíaca en dermatitis herpetiforme. ¿Cuál es la mejor opción para detectar sensibilidad al gluten?

Background: Dermatitis herpetiformis (DH), a wellestablished gluten-sensitive skin disorder presenting variable degrees of enteropathy, constitutes a very useful model in order to assess the utility of the celiac disease (CD)-related serology in patients with mild intestinal damage. Objective: Our aim was to explore comparatively the performance of a panel of CD-related serologic tests in patients with DH. Methods: We assessed a series of 18 consecutive patients with skin biopsy proven DH presenting the overall spectrum of intestinal damage ranging from normal mucosa (n=6) to total villous atrophy (TVA) (n=6) through partial villous atrophy (PVA) (n=6). Sera were obtained from all patients while consuming a gluten containing diet. Serologic tests were antiendomysial, anti-tissue transglutaminase and antigliadin antibodies, and newly developed tests detecting both antibody isotypes (IgA and IgG) against deamidated synthetic gliadin-derivedpeptides (a-GDP). Results: Serologic tests had a variable behaviour depending on the degree of enteropathy. While the majority of tests detected patients with TVA, only 50% of those with normal histology had positive assays. Patients with PVA had discordant results. Classical CD-specific tests were positive in only some patients with mild damage while all of them were identified by a single assay detecting both isotypes of a-GDP. Conclusion: The detection of a-GDP antibodies was the most reliable tool in order to identify gluten sensitivity in DH patients presenting a wide range of intestinal damage. Further studies should explore if these findings can be extrapolated to patients with CDhaving mild enteropathy.

Introducción: la dermatitis herpetiformis (DH), una lesión dermatológica consecuencia de sensibilidad al gluten y asociada a grados variables de enteropatía, constituye un modelo muy útil con el objeto de evaluar la eficacia de la serología de la enfermedad celíaca(EC) en pacientes con daño intestinal leve. Objetivo: explorar comparativamente la utilidad de una serie de anticuerpos empleados en EC en pacientes con DH. Métodos: analizamos una serie de 18 pacientes consecutivos con diagnóstico de DH por biopsia de piel que presentaban el más amplio espectro de daño intestinal variando desde una mucosa normal (n=6) a la atrofia vellosa total (AVT) (n=6) y pasando por atrofia vellosaparcial (AVP) (n=6). Se obtuvo plasma de todos los pacientes mientras consumían gluten. Las pruebas serológicas empleadas fueron anticuerpos antiendomisio, anti-transglutaminasa y atigliadina, y unas pruebas recientemente desarrolladas que detectan anticuerpos IgA e IgG dirigidos contra péptidos sintéticos deamidados derivados de la gliadina (a-GDP). Resultados: las diferentes pruebas tuvieron un comportamiento variable dependiendo del grado de lesión intestinal. Mientras que la mayoría de las pruebas detectaron a todos los pacientes con AVT, sólo el 50% de aquellos con histologíanormal tuvieron resultados positivos. Los pacientes con AVP tuvieron resultados discordantes. Así las pruebas clásicas fueron positivas en sólo algunos pacientescon daño leve, mientras que todos ellos fueron positivos a una prueba para detectar ambos isotipos del a-GDP. Conclusión: la determinación de anticuerpos a-GDP fue la herramienta más confiable con el objeto de identificar serológicamente la sensibilidad al gluten en pacientes con DH que presentan variables grados de daño intestinal. Otros estudios deberían explorar si estos hallazgos podrían ser extrapolados a pacientes conEC con enteropatía de grado leve.

Manifestaciones cutáneas de las enfermedades gastrointestinales: primera parte / Cutaneous manifestations of gastrointestinal diseases: first part

Es de gran importancia que tanto los dermatólogos como los gastroenterólogos estén familiarizados con los hallazgos cutáneos que se pueden presentar antes, durante la evolución o como complicaciones de diversas enfermedades del sistema digestivo. A continuación se presenta el primero de dos artículos de revisión de las principales manifestaciones dermatológicas de las enfermedades gastrointestinales, donde se enfatiza el hecho de que los hallazgos cutáneos muchas veces son la clave para realizar un diagnóstico de los trastornos gastrointestinales y de sus complicaciones, por lo que es posible instaurar precozmente medidas preventivas y terapéuticas.

Epidermólise bolhosa / Epidermolysis bullosa

Os autores fazem uma revisäo da literatura sobre essa entidade e relatam 3 casos ocorridos no HU-UFJF no período de 1992 e 1998 colocando em evidência as principais características clínicas encontradas.

Alteraciones de la zona de membrana basal en enfermedades cutáneas / Abnormalities of the basement membrane zone in cutaneous diseases

Diversas enfermedades cutáneas se caracterizan por alteraciones localizadas preferentemente en la zona de membrana basal. Estas dermatosis se han hereditarias y adquiridas. Se pueden encontrar cambios ultraestructurales en la membrana basal, muchos de los cuales serían causados por fenómenos autoinmunes. Múltiples investigaciones han permitido aclarar la morfogénesis de las lesiones clínicamente visibles que afectan esta zona en particular. En la presente revisión se realiza una puesta al día de la última información de la literatura sobre estas interesantes enfermedades